Affiliations: [a]
Neuroscience Research Australia, Sydney, Australia
| [b]
ARC Centre of Excellence in Cognition and its Disorders, Sydney, Australia
| [c]
School of Medical Sciences, The University of New South Wales, Sydney, Australia
| [d]
Institute Born-Bunge, University of Antwerp, Antwerp, Belgium
| [e] Faculty of Health Sciences, The University of Sydney, Sydney, Australia
Correspondence:
[*]
Correspondence to: Dr. Tim Van Langenhove, Neuroscience Research Australia, Margarete Ainsworth Building, Barker Street (PO Box 1165), Randwick NSW 2031, Australia. Tel.: +61 2 9399 1134; Fax: +61 2 9399 1047; E-mail: tim.vanlangenhove@gmail.com
Abstract: Background: Differentiating between primary progressive aphasia (PPA) variants based on the profile of language deficits can be difficult in a proportion of patients. Further, little is presently know about the pattern of longitudinal changes in behavior in PPA variants. Objective: To determine the presence of behavioral changes in the main variants of PPA: semantic (sv-PPA), nonfluent/agrammatic (nfv-PPA), and logopenic (lv-PPA), and establish the course of these changes over time. Methods: We measured behavioral changes in 73 prospectively recruited PPA (30 sv-PPA, 22 nfv-PPA, and 21 lv-PPA), as well as 33 behavioral variant frontotemporal dementia (bv-FTD) and 31 Alzheimer’s disease (AD) patients, at baseline and after 1 year, using the Cambridge Behavioural Inventory Revised. All included patients had mild dementia severity at baseline. Results: Both at baseline and follow-up, sv-PPA exhibited significantly more behavioral disturbances of the type characteristic of bv-FTD compared with other PPA variants. 74% of sv-PPA patients with mild dementia severity exhibited at least one behavior disturbance at baseline, which increased to 84% during follow-up. Behavioral symptoms did not differ between nfv-PPA and lv-PPA groups at baseline. At follow-up, however, empathy loss was significantly more pronounced in nfv-PPA. The prevalence and course of behavioral symptoms in lv-PPA was similar to that found in AD. Conclusions: sv-PPA show more prominent FTD-like behavioral disturbances compared with other PPA variants which typically emerge already early in the disease course. Empathy loss may be an important factor that helps differentiating nfv-PPA from lv-PPA. Our results may allow improved prediction of likely progression in behavioral symptoms across the PPA variants.
